CASE REPORT
Filiz VURAL1, Guray SAYDAM1, Fahri SAHIN1, Nur A. SOYER1, Mine HEKIMGIL2,
Serkan OCAKCI1, Murat TOMBULOGLU1, Seckin CAGIRGAN1
1
SUMMARY
Non-Hodgkins lymphoma (NHL) of the breast can be either primary or secondary. Both are rare diseases accounting less than 0.6%
of all breast malignancies. In this study we report our experience with five cases of breast lymphoma, 3 of them being primary and
2 of them being secondary breast lymphoma, retrospectively. All patients were female with a median age of 47 years and presented with breast masses (3 in the right breast and 2 in the left). The histologic subtype showed broad spectrum, one patient had peripheral T-cell lymphoma and 4 patients had B-cell lymphomas; 2 were diffuse large B-cell lymphomas, 1 was a diffuse small B-cell
lymphoma, and the last patient was a marginal zone lymphoma. Partial mastectomy was performed in 2 patients to carry out diagnosis. Anthracycline-based chemotherapy regimens were employed in 4 patients. Radiotherapy was added to other treatment modalities in 3 cases. At a median follow-up of 5 years, all patients were alive other than one who had been diagnosed with peripheral T-cell lymphoma just after pregnancy and showed resistance to the applied treatment. The treatment and the prognosis of breast lymphomas were not different from the currently indicated for nodal NHL with the same stage and histological subtype.
Keywords: Breast mass, Lymphoma
ZET
Meme Non-Hodgkin Lenffomalar: Be Olgu Sunumu ve Literatrn Gzden Geirilmesi
Memede saptanan Non-Hodgkin lenfomalar primer ya da sekonder olabilir. Her ikisi de olduka nadir olup, meme kanserlerinin yaklak %0.6sn olutururlar. Bu almada, kliniimizde izlenen 3 primer, 2si sekonder meme lenfomas olan olgular geriye dnk
olarak inceleyerek kendi tecrbelerimizi bildirmek istedik. Btn hastalar kadnd ve ortalama ya 47 idi. Btn hastalarda memede
kitle ikayeti vard (3 hastada sa, 2 hastada sol). Histolojik alt tipleri geni bir spektruma sahipti; 1 hastada periferal T hcreli lenfoma, 4 hasta B hcreli lenfoma (2 hasta diffz byk B-hcreli, 1 hasta diffz kk B-hcreli, 1 hasta marjinal zon lenfoma)
olarak tan ald. ki hastada tansal amal parsiyel mastektomi yapld. 4 Hastada antrasiklin esasl kemoterapi rejimi uyguland.
hastada tedaviye radyoterapi eklendi. Ortalama 5 yllk takip sonras, gebelikten hemen sonra periferal T hcreli lenfoma tans alan ve
kemoterapiye direnli olan olgu dndaki dier 4 olgu hayattayd. Meme lenfomalarnn tedavi ve prognozlar, ayn evre ve histolojik
tipteki nodal lenfomalardan farkl deildir.
Anahtar Kelimeler: Memede kitle, Lenfoma
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doi: 10.4999/uhod.10023
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INTRODUCTON
Breast involvement by malignant lymphoma, whether primary or secondary, is a rare event.1 It accounts
for 2.2% of all extranodal lymphomas and less than
0.5% of all malignant breast tumors.1-6 Primary and
secondary lymphomas of the breast are defined according to the criteria used for other extranodal
lymphomas. Primary breast lymphoma (PBL) was
diagnosed when the breast was the site of first or major manifestation of the lymphoma and there was no
documentation of lymphoma elsewhere, excluding
the presence of ipsilateral axillary node involvement
by Wiseman and Liaos definition.7 However, these
criteria are overly restrictive, since they limit the definition of PBLs to those exclusively localized to the
breast. Patients with lymphoma of the breast that have disseminated elsewhere before diagnosis are not
accepted. All lymphomas involving the breast but
not including these criteria are considered as secondary breast lymphomas (SBLs). Pathologically there
are no differences between primary or secondary
lymphomas of the breast.2
Here we present the clinical and pathological features, treatment outcomes of 5 cases with extranodal
lymphomas involving the breast that were followedup at our hematology unit, retrospectively.
CASE 1
A 50-year-old woman was admitted to hospital with
the complaint of mass in her right breast and axillary
region. She denied fever, night sweats and weight
loss. On physical examination, the right breast was
edematous and a painless mass of 5x5 cm was present in the upper lateral quadrant of the ipsilateral
breast. Bulky lymphadenopaty (LAP) package was
also palpated in the right axilla. Left breast and axilla were normal. Mammography of both breasts showed a mass measuring 5x5 cm in upper outer quadrant of the right breast. Histopathological and immunohistochemical examination of the excisional biopsy material revealed diffuse large B-cell nonHodgkins lymphoma (DLBCL). Computed tomographic (CT) examination of neck, thorax and abdomen and bone marrow biopsy showed that there were no further lymphoma infiltrations. She was classified as stage IIE according to Ann Arbor classification system and treated with 8 cycles of cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)
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chemotherapy. After 8 cycles of R-CHOP chemotherapy she achieved CR. She has been under out-patient follow-up for four years without relapse.
CASE 4
A 23-year-old woman was admitted with a painless
lump in the left breast and the left neck appearing
just after terminating pregnancy. On physical examination, the lump in the upper outer quadrant of the
left breast was found to be painless and immobile.
There was no skin retraction, nipple discharge or peau dorange appearance. There were conglomerated
LAPs on both sites of the supraclavicular area and in
the left axilla. Hematological and biochemical examination of the blood including lactate dehydrogenase (LDH) level were normal. A fine needle aspiration biopsy from the lump of left breast confirmed the
diagnosis of peripheral T-cell lymphoma. Bone marrow biopsy was normal. CT of the whole body showed the presence of bilateral paratracheal LAPs, in
addition to left axillary, bilateral supraclavicular
LAPs, and a mass in the upper outer quadrant of the
left breast measuring 10x15 cm. The patient had received 5 cycles of CHOP chemotherapy but the tumor showed resistance to treatment. Then, she was
treated with 6 cycles of mechloretamine, vincristine,
procarbazine, prednisone (MOPP) chemotherapy followed by mantle radiotherapy with total dose of 3600
cGy, and achieved CR. Two years after the CR she
relapsed with the left cervical and supraclavicular
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CASE 5
A 47-year-old woman was referred to our hematology unit with a mass in the right breast and right
neck noticed during self examination 15 days before
admission. Mammography of both breasts showed
the presence of 4 different masses the largest measuring 5x3.5cm in the upper outer quadrant of the right
breast. Multiple LAPs were detected with CT in bilateral axillary, cervical and supraclavicular areas. CT
analysis also confirmed the presence of multiple
LAPs making packages in mediastinum and abdomen. She underwent partial mastectomy and masses
were all extracted. Pathological and immunohistochemical analysis revealed a marginal zone nonHodgkins lymphoma (Figure 1). She has had night
sweats for a year, but denied fever and weight loss.
Physical examination of the patient revealed bilateral
supraclavicular, cervical and axillary LAPs, and
scars due to the operation in right breast. Bone marrow examination showed lymphoma infiltration with
immunohistochemical staining (CD20+, CD45RO-,
CD5-, CD23-, TdT-, cyclin D1-, CD10-) suggesting
a B-cell marginal zone lymphoma (Figure 2). The di197
agnosis of a stage IV-B marginal zone NHL according to Ann Arbor clinical staging system was established. After 6 cycles of CHOP chemotherapy, residual lymphoma infiltration in the bone marrow still
remained. Treatment of the patient continued with 2
cycles of etoposide, solumedrol, high-dose cytosine
arabinoside, cisplatin (ESHAP) chemotherapy regimen and she achieved complete remission. However,
6 months later, bone marrow examination showed
lymphoma infiltration once again. Bulky abdominal
mass was also detected with CT analysis at the same
time. Radiotherapy at a dose of 5000 cGy to paraaortic LAPs in abdomen was administered. After the last
therapy CT examination of the neck, thorax and abdomen were all normal. She received 4 cycles of rituximab (375 mg/m2) and dexamethasone, high-dose
cytosine arabinoside, cisplatin (DHAP) chemotherapy. Residual lymphoma infiltration in bone marrow
was detected in every bone marrow biopsy examination. She is alive with disease for six years, taking
low-dose oral chlorambucil, and rituximab infusions
once in every up to 8 cycles.
DISCUSSION
Clinicopathologic features and treatment outcomes
of 5 women with breast lymphomas diagnosed and
followed-up in Ege University Adult Hematology
Unit were evaluated retrospectively. Median age of
patients was 47 years (range 23-65 years) representing the literatures information; the median age of
patients diagnosed with breast lymphoma either primary or secondary is between 40 and 67 years, but
the range is broad. These tumors can appear in teenagers or patients in their 90s, but the peak age incidence is during the six decade.3,8-10
Extranodal NHL accounts for 10-48% of all NHL cases. Commonly involved extranodal sites include the
stomach, tonsils, lungs, adenoids, skin, small intestine, and testis.1,3,8,11,12 Lymphoma involving the breast
is very rare. The rarity of the breast lymphoma may
be related to the relatively small amount of lymphoid tissue present in the breast as compared to the gut
or lung in which primary lymphomas are much more
frequent.8,11-14 All published series reported an overwhelming female predominance.3,8,9 It has been reported that right breast is involved more common than
the left breast.3,13,15 Bilateral breast involvement at
presentation or relapse in the contralateral breast is
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primary, 2 of them having secondary breast lymphomas. At a median follow-up of 5 years, all 3 patients
with primary disease achieved CR, and remained in
CR for median 3 years. Prognosis and treatment results were not different from the other lymphomas
with the same histopathological classification. Primary breast lymphomas (PBLs) according the described criteria7 are stage I and II, so having good prognosis compared to secondary breast lymphoma are
inevitable. According to our observation, the treatment modalities of lymphomas involving the breast
should not be different than the other lymphomas
with the same stage and histological classification.
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Cohen PL, Brooks JJ. Lymphomas of the breast. A clinicopathologic and immunohistochemical study of primary and secondary cases. Cancer 67: 1359-1369,
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Bobrow LG, Richards MA, Happerfield LC, et al. Breast lymphomas: a clinicopathologic review. Hum Pathol 24: 274-278, 1993.
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Correspondence:
Dr. Gray SAYDAM
Ege niversitesi Tp Fakltesi
Hematoloji Anabilim Dal
Bornova, ZMR / TURKEY
Tel-fax: (+90.232) 390 35 30
guray.saydam@ege.edu.tr
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